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[4] Nonstandard abbreviations: LSD, lysosomal storage disorders; DBS, dried blood spots; MS/MS, tandem mass spectrometry; TPP1, tripeptidyl peptidase 1; NAGLU, [alpha]-N-acetylglucosaminidase; GUSB, lysosomal [beta]-glucuronidase; IUDA, [alpha]-iduronidase; MPSs, mucopolysaccharidoses; I2S, iduronate-2-sulfatase; GALNS, N-acetylgalactosamine- 6sulfatase; ARSB, N-acetylgalactosamine-4-sulfatase; LINCL, infantile neuronal ceroid lipofuscinosis; ESI, electrospray ionization; LC, liquid chromatography; P, product; IS, internal standard; S, substrate; MRM, multiple reaction monitoring.

Mean activity ([micro]mol/h/L blood) (a) Sample GALNS NAGLU ARSB Blank (n = 6) 0.0033 0.01 0.05 CDC QC low (b) (n = 2) 0.17 0.23 0.46 CDC QC high (b) (n =2) 3.0 5.8 5.1 Adults (n = 4) 1.1 4.1 1.2 Newborns (n = 62) 0.67 1.6 4.4 MPS IIIB patients (n =4) 0.83 0.01 1.7 MPS VII patients (n =3) 1.0 3.2 1.8 LINCL patients (n = 7) 1.2 3.1 1.8 Mean activity ([micro]mol/h/L blood) (a) Sample I2S GUSB TPP1 Blank (n = 6) 0.06 0.06 0.03 CDC QC low (b) (n = 2) 0.98 13.0 5.0 CDC QC high (b) (n =2) 14.6 44.4 22.7 Adults (n = 4) 17.0 19.5 19.0 Newborns (n = 62) 16.1 28.5 35.9 MPS IIIB patients (n =4) 19.2 17.7 21.2 MPS VII patients (n =3) 26.1 0.08 20.4 LINCL patients (n = 7) 17.1 27.0 1.3 (a) For SDs in these measurements see online Supplemental Table 2.

GALNS showed the lowest mean activity at 0.67 [micro]mol/h/L blood followed by NAGLU (1.56 [micro]mol/h/L blood), ARSB (4.37 [micro]mol/h/L blood), I2S (16.1 [micro]mol/h/L blood), and GUSB (28.5 [micro]mol/h/L blood).

Multiplex Tandem Mass Spectrometry Enzymatic Activity Assay for Newborn Screening of the Mucopolysaccharidoses and Type 2 Neuronal Ceroid Lipofuscinosis

For 16 aerobic isolates representing 10 RFLP groups, and 7 anaerobes representing 4 RFLP groups, plasmid and chromosomal DNA were screened for the presence of arsA, arsB, and arsC (Figure 5; Table 2).

Because the genes are part of the same operon and are regulated together, the absence of observed arsB or arsC along with other ars genes is probably indicative of variations in the gene that decreased the homology with our primer set.

Use of ecotoxicological tools to evaluate the health of New Bedford Harbor sediments: a microbial biomarker approach

"To further ensure adequate servicing of ongoing profit obligations, the transaction imposes minimum performance covenants on ARSB (as the Issuer) and Axis REIT (as the third-party security provider).

RAM Ratings reaffirms ratings of Axis REIT Sukuk's MYR 110 million first Sukuk Issue

As part of the trigger mechanisms, the transaction also imposes minimum performance covenants on ARSB and Axis REIT.

RAM Ratings reaffirms ratings of Axis REIT's MYR 155 million Second Sukuk

ARSB will subsequently utilise these payments to meet the Periodic Profit Payments (PPP) on the Sukuk.

Distinguishing features of this transaction include minimum performance covenants at both ARSB and Axis REIT levels, where failure to meet pre-determined coverage levels will constitute a trigger event.

RAM Ratings assigns final ratings to Axis REIT Sukuk's second issue